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With rare disease threatening mother, unborn baby, Chicago doctors perform remarkable procedure to save both

A mother and baby in crisis. A rare genetic disease threatens both of their lives. That’s when doctors stepped in and performed surgery in the second trimester of pregnancy. A frightening ordeal and tonight a happy ending.

She’s precious. This is Luna Paisley – born full-term and healthy.

Iyana Baker, Marfan syndrome patient: “I feel stronger, and it’s a nice story to tell her when she gets older.”

Thankfully, Iyana Baker’s story has a happy ending.

Iyana Baker: “My whole life everyone said I shouldn’t have babies, shouldn’t get pregnant. I was a high risk patient.”

That’s because Iyana has Marfan syndrome – a disorder that causes the connective tissue in the body to stretch, namely the aorta – the main blood vessel that supplies blood to the body. Her mother and grandmother had it, as well.

Iyana Baker: “They knew I had Marfan syndrome so they wanted to check on my heart.”

Iyana was well into her pregnancy and undergoing a routine fetal ultrasound when – given her family history -- the technologist decided to check the young mother’s heart.

Iyana Baker: “They checked it and saw I had a splitting in my aorta. It’s been splitting over time.”

The walls of Iyana’s aorta were weakened and dilated – a rupture was imminent.

Dr Robert Gordon, cardiologist, NorthShore University HealthSystem: “This line right here is her dissection. It is a life-threatening situation.”

Dr Hyde Russell, cardiac surgeon, NorthShore University HealthSystem: “If you have a patient who is pregnant and they are gaining fluid all the way during their pregnancy, the volume load of pregnancy only increases the stress of the aorta. She’s at risk for the aorta to tear more, to rupture, for the baby to have a problem, all of the above.”

Iyana was admitted to the hospital immediately as NorthShore University HealthSystem cardiac surgeon Dr Hyde Russell formed a plan to repair the severely damaged aorta – all while keeping his patient and her growing baby alive.

Dr Hyde Russell: “The main thing is just blood flow to the baby. You have to make sure there is enough blood flow to the baby during the operation. One thing that is commonly done when heart surgeons operate on someone is they cool them down somewhat. You can’t do that in this case. You have to keep the temperature at a normal level for the baby otherwise the heart of the baby will have a problem. The other thing is electrolyte abnormalities that can occur during heart surgery. You have to be very careful. The potassium levels and so on can really affect the baby’s heart rate. While you are doing this you’re thinking about all these little things.”

But throughout it all, Iyana was thinking about her little one.

Iyana Baker: “I just think thinking positive really helps. I was still really positive about it.”

Dr Russell: “This is a dacron graft.”

During the four-plus hour operation that included experts from maternal and fetal medicine, intensive care and anesthesiology, Dr Russell removed the damaged portion of Iyana’s aorta and sewed in a replacement graft.

Dr Hyde Russell: “In the end this is where the graft sits. It comes to the heart and then goes up to supply the brain and down the descending aorta to supply the kidneys, the liver, the legs. It doesn’t dilate, doesn’t rupture. Once it’s done you are good for life.”

About four months later … a new life began.

Iyana Baker: “Nothing bad happened. You didn’t come out early or anything. I was pretty confident that everything was going to be ok. It turned out better than I thought it was. Now I feel like I’m invincible because I went through so much.”

She certainly did, but Iyana will have to be monitored her entire life to make sure her aorta stays healthy. Baby Luna will be watched closely, as well, and likely undergo genetic testing to see if she carries the same risk her mother does.